Table 1.
Patient characteristics
| Diagnosis | P‐value | |||||
|---|---|---|---|---|---|---|
| Control (n = 19) | SSc (n = 38) | SSc‐PAH (n = 19) | P‐valueb | P‐valuec | P‐valued | |
| Gender; Female | 68% | 87% | 68% | 0·1 | 1·0 | 0·1 |
| Age | 59 ± 15 | 57 ± 11 | 64 ± 13 | 0·8 | 0·4 | 0·1 |
| BSA (m2) | 1·8 ± 0·2 | 1·8 ± 0·2 | 1·8 ± 0·2 | 0·7 | 1·0 | 0·9 |
| NIBPs (mmHg) | 128 ± 12a | 130 ± 22 | 123 ± 18 | 0·9 | 0·7 | 0·4 |
| NIBPd (mmHg) | 76 ± 9a | 74 ± 9 | 73 ± 11 | 0·8 | 0·7 | 1 |
| CMR | ||||||
| LVEF (%) | 58 ± 5 | 62 ± 6 | 50 ± 9 | 0·1 | 0·001 | <0·001 |
| HR (/min) | 62 ± 8 | 74 ± 9 | 87 ± 13 | <0·001 | <0·001 | <0·001 |
| LVEDVI (mL m−2) | 87 ± 14 | 75 ± 14 | 64 ± 13 | 0·005 | <0·001 | 0·02 |
| LVESVI (mL m−2) | 37 ± 8 | 29 ±8 | 32 ± 9 | 0·004 | 0·2 | 0·3 |
| LVSVI (mL m−2) | 50 ± 8 | 45 ± 9 | 31 ± 8 | 0·08 | <0·001 | <0·001 |
| CI (L min−1 m−2) | 3·1 ± 0·6 | 3·3 ± 0·7 | 2·7 ± 0·7 | 0·5 | 0·2 | 0·006 |
| RVEF (%) | 57 ± 5 | 60 ± 7 | 38 ± 10 | 0·2 | <0·001 | <0·001 |
| RVEDVI (mL m−2) | 91 ± 20 | 73 ± 13 | 100 ± 28 | 0·02 | 0·4 | <0·001 |
| RVESVI (mL m−2) | 40 ± 11 | 29 ± 9 | 63 ± 24 | 0·03 | <0·001 | <0·001 |
| RVSVI (mL m−2) | 52 ± 11 | 44 ± 6 | 37 ± 9 | 0·002 | <0·001 | 0·03 |
| RHC | N = 9 | N = 18 | ||||
| sPAP (mmHg) | 31 ± 7 | 65 ± 19 | <0·001 | |||
| mPAP (mmHg) | 18 ± 3 | 39 ± 11 | <0·001 | |||
| PAWP (mmHg) | 7 ± 4 | 5 ± 3 | 0·1 | |||
| mRAP (mmHg) | 2 ± 1 | 5 ± 5 | 0·1 | |||
| PVR (wood units) | 2 ± 1 | 7 ± 4 | <0·001 | |||
| CI (L min−1 m−2) | 3·1 ± 0·6 | 3·1 ± 0·8 | 0.9 | |||
| Comorbidities | ||||||
| Smoker; yes/ex | 13/29% | 5/53% | 0·2 | |||
| Diabetes; yes | 13% | 16% | 0·8 | |||
| Raynaud's | 100% | 100% | 1 | |||
| Limited/diffuse SSc | 79/21% | 95/3% | 0·1 | |||
| mRSS | 5 ± 9 | 1 ± 2 | 0·03 | |||
| IHD | 5% | 8% | 0·7 | |||
| SSc duration | 4·3 ± 6·8 | 4·8 ± 5·0 | 0·8 | |||
| ACE/ARB | 11% | 21% | 0·3 | |||
| CCB | 39% | 47% | 0·6 | |||
| BB | 5·3% | 5·3% | 1 | |||
| Statin | 2·6% | 11% | 0·2 | |||
| ERA | 0% | 63% | <0·001 | |||
| PDEI5 | 0% | 26% | 0·001 | |||
| Prostanoid | 0% | 5·3% | 0·2 | |||
| NSAID | 5·3% | 16% | 0·2 | |||
| Corticosteroid | 13% | 21% | 0·4 | |||
| Immunosuppressant | 7·9% | 16% | 0·4 | |||
Control, healthy adult volunteers; SSc‐PAH, systemic sclerosis with pulmonary arterial hypertension; SSc, systemic sclerosis without pulmonary arterial hypertension; BSA, body surface area; NIBPs, non‐invasive blood pressure, systolic; NIBPd, non‐invasive blood pressure, diastolic; CMR, cardiac magnetic resonance imaging; LVEF, left ventricle ejection fraction; HR, heart rate; LVEDVI, left ventricle end‐diastolic volume index to BSA; LVESVI, left ventricle end‐systolic volume index; LVSVI, left ventricle stroke volume index; RVEDVI, right ventricle end‐diastolic volume index; RVESVI, right ventricle end‐systolic volume index; RVSVI, right ventricle stroke volume index; CI, cardiac index; RHC, right heart catheterization; sPAP, systolic pulmonary artery pressure; mPAP, mean pulmonary artery pressure; PAWP, pulmonary artery wedge pressure; mRAP, mean right atrial pressure; PVR, pulmonary vascular resistance; mRSS, modified Rodnan skin score; IHD, ischaemic heart disease; SSc duration in years; ACE/ARB, Ace inhibitor/angiotensin II blocker; CCB, dihydropyridine calcium channel blocker; BB, beta blocker; ERA, endothelin receptor antagonists; PDEI5, phosphodiesterase type 5 inhibitor; NSAID, non steroidal anti‐inflammatory drugs.
n = 17.
P‐value: comparison between control and SSc.
P‐value: comparison between control and SSc‐PAH.
P‐value: comparison between SSc and SSc‐PAH.